MyMedR

Macrophage activation syndrome triggered by systemic lupus erythematosus flare: successful treatment with a combination of dexamethasone sodium phosphate, intravenous immunoglobulin, and cyclosporine: a case report

Gouda W, Alsaqabi F, Moshrif A, Abbas AS, Abdel-Aziz TM, Islam MA

J Med Case Rep, 2021 Oct 07;15(1):497.

PMID: 34620236 DOI: 10.1186/s13256-021-03072-1

BACKGROUND: Macrophage activation syndrome is classified as a secondary form of hemophagocytic lymphohistiocytosis. It is a hyperinflammatory complication observed to be comorbid with a variety of autoimmune diseases, including adult-onset Still's disease and systemic juvenile idiopathic arthritis. Macrophage activation syndrome is less commonly detected in adult patients with systemic lupus erythematosus, which, if untreated, can be fatal, though determining the optimum treatment strategy is still a challenge.

CASE PRESENTATION: Herein, we report a case of macrophage activation syndrome in a 33-year-old Egyptian female as an unusual complication of a systemic lupus erythematosus flare in adult patients. Our patient was initially treated with a combination of intravenous methylprednisolone pulse therapy and intravenous immunoglobulin therapy, which was followed by a course of oral prednisolone and oral cyclosporine with little response. Switching from oral prednisone to intravenous dexamethasone sodium phosphate showed a more favorable clinical and biochemical response.

CONCLUSION: Macrophage activation syndrome is less commonly detected in adult patients with systemic lupus erythematosus. Our case demonstrates that dexamethasone sodium phosphate can be a successful alternative treatment for patients with systemic lupus erythematosus complicated by macrophage activation syndrome in whom the response to pulse methylprednisolone was inadequate to manage their illness, proving to be remarkably effective in a relatively short time frame.

Matched MeSH terms: Cyclosporine/therapeutic use

Fulltext Missed perforation presenting as sympathetic ophthalmia in an adolescent Malay girl

Bastion ML, Mohamad MH

BMJ Case Rep, 2012;2012.

PMID: 22914237 DOI: 10.1136/bcr-2012-006525

To describe the rare presentation of sympathetic ophthalmia in a teenage girl with no previous known ocular injury.

Matched MeSH terms: Cyclosporine/therapeutic use

Haemophagocytic lymphohistiocytosis in Malaysian children

Ariffin H, Lum SH, Cheok SA, Shekhar K, Ariffin WA, Chan LL, et al.

J Paediatr Child Health, 2005 Mar;41(3):136-9.

PMID: 15790325

To study the clinical presentation, therapy and outcome of children diagnosed with both primary and secondary haemophagocytic lymphohistiocytosis (HLH) at the University of Malaya Medical Centre.

Matched MeSH terms: Cyclosporine/therapeutic use*

Renal transplantation in Malaysia

Fan KS, Lim TO, Morad Z, Suleiman AB, Lei CC, Khairullah A

Transplant Proc, 1995 Feb;27(1):1466-8.

PMID: 7878944

Matched MeSH terms: Cyclosporine/therapeutic use

A randomized, controlled trial to assess the safety of switching stable renal transplant patients from Sandimmun to Sandimmun Neoral

Loo CS, Morad Z, Lim TO, Fan KS, Suleiman AB

Transplant Proc, 1996 Jun;28(3):1328-9.

PMID: 8658680

Matched MeSH terms: Cyclosporine/therapeutic use*

Impact of cyclosporine dose on renal allograft function in an Asian population

Vathsala A, Lee WT, Lim CH

Transplant Proc, 1994 Oct;26(5):2507-8.

PMID: 7940768

Matched MeSH terms: Cyclosporine/therapeutic use*

Conversion from Sandimmune to Neoral in patients with stable renal allograft function: UHKL experience

Goh BL, Jalil R, Koh SN, Chua CT, Tan SY

Transplant Proc, 1998 Nov;30(7):3535-6.

PMID: 9838548

Matched MeSH terms: Cyclosporine/therapeutic use

FK506 rescue therapy for acute renal allograft rejection

Goh BL, Morad Z, Cheah PL, Chua CT, Tan SY

Transplant Proc, 1998 Nov;30(7):3592-3.

PMID: 9838574

Matched MeSH terms: Cyclosporine/therapeutic use

Renal function following liver transplantation for unresectable hepatoblastoma

Lee WS, Grundy R, Milford DV, Taylor CM, de Ville de Goyet J, McKiernan PJ, et al.

Pediatr Transplant, 2003 Aug;7(4):270-6.

PMID: 12890004

Combination of cyclosporine (CsA) and tacrolimus immunosuppression post-liver transplantation (LT) and the chemotherapeutic drugs used to treat hepatoblastoma (HB), are nephrotoxic. We aimed to determine the severity and duration of nephrotoxicity in children following LT for unresectable HB. We reviewed all children undergoing LT for unresectable HB at the Liver Unit, Birmingham Children's Hospital, UK, from 1991 to July 2000. Thirty-six children undergoing LT for biliary atresia, matched for age and sex, were selected as controls to compare pre- and post-LT renal function. Renal function was determined by estimation of glomerular filtration rate (eGFR) derived from plasma creatinine using Schwartz's formula. Twelve children with HB (mean age of diagnosis 33 months) who underwent LT (mean age 47 months) and 36 controls (mean age of LT 34 months) were studied. CsA was the main immunosuppressive drug used in each group. The median eGFR before, and at 3, 6, 12, 24 and 36 months after LT in HB group was significantly lower than controls (93 vs. 152, 66 vs. 79, 62 vs. 86, 66 vs. 87, 64 vs. 94, 53 vs. 90 mL/min/1.73 m2, respectively; 0.01 < p < 0.03). The reductions in the median eGFR of both the HB group and controls before and at 36 months after LT were 49 and 41%, respectively. At 36 months after LT, there was a trend for partial recovery of the eGFR in the controls but not in the HB group. Children who underwent LT for unresectable HB had renal dysfunction before transplantation that persisted for 36 months after LT.

Matched MeSH terms: Cyclosporine/therapeutic use

Fulltext Case of severe refractory myasthenia gravis in HUKM

Hamizah R, Norlinah MI, Tan HJ, Soehardy Z, Halim AG, Rohana AG, et al.

Med J Malaysia, 2006 Dec;61(5):633-5.

PMID: 17623968 MyJurnal

A 20-year-old girl first notice bilateral ocular muscle weakness in 2001. Two months later, she developed acute muscle paralysis and respiratory failure which required ventilation. Serum anti-acetylcholine receptor antibodies and repetitive nerve stimulation test was positive and consistent with myasthenia gravis (MG). CT scan thorax revealed thymic enlargement and she underwent a video assisted thymectomy (VATS). However, over the next three years, despite maximal doses of various immunosuppressive agents with plasmapheresis and intravenous immunoglobulin, she was admitted with recurrent myasthenic crisis without any obvious precipitant. She was then commenced on mycophenolate mofetil and together with regular plasmapheresis, cyclosporine and prednisolone, her symptoms have finally improved and brought under control.

Matched MeSH terms: Cyclosporine/therapeutic use

Neoral (cyclosporine) C2 monitoring in renal transplant recipients: a single-center experience in Asia

Wong HS, Morad Z

Transplant Proc, 2003 Feb;35(1):230-1.

PMID: 12591376

Matched MeSH terms: Cyclosporine/therapeutic use

FK506 "rescue" therapy complicated by renal tubular acidosis in renal allograft recipients

Goh BL, Tan SY

Transplant Proc, 1998 Nov;30(7):3594-5.

PMID: 9838575

Matched MeSH terms: Cyclosporine/therapeutic use

Population Pharmacokinetic Modeling of Cyclosporine Among Malaysian Renal Transplant Patients: An Evaluation of Methods to Handle Missing Doses in Conventional Drug-Monitoring Data

Albitar O, Ballouze R, Harun SN, Mohamed Noor DA, Sheikh Ghadzi SM

J Clin Pharmacol, 2020 11;60(11):1474-1482.

PMID: 32557653 DOI: 10.1002/jcph.1670

Cyclosporine is a primary drug in transplant immunosuppression regimens. It has a narrow therapeutic index and variable pharmacokinetic behavior. This study aimed to develop a population pharmacokinetic model of cyclosporine in Malaysian renal transplant recipients as well as to evaluate the performances of different methodsfor handling missing doses. A total of 2804 concentrationts predose and 2 hours after doses were collected retrospectively from 113 renal transplant patients on cyclosporine in Penang General Hospital. Model structure and pharmacokinetic parameters were estimated using nonlinear mixed-effects modeling software. Missing doses were handled using different methods to evaluate their performance. Covariate analysis was performed using stepwise forward addition (P < .05) followed by backward elimination (P < .001). Prediction-corrected visual predictive check and sampling-importance resampling methods were used to validate the final model. A 1-compartment model with first-order absorption and elimination best fitted the data. All methods to handle missing doses performed well with the missing dose method being superior to other methods and thus was applied in the final model. Cyclosporine clearance (CL/F) was estimated as 15.1 L/h, and volume of distribution (V/F) was 108 L. Postoperative time, sex, and calcium channel blockers were identified as significant covariates on CL/F, whereas sex and cholesterol level were identified as significant covariates on V/F. This is the first population pharmacokinetic model developed in Malaysian renal transplant patients using a large sample with an evaluation of different methods to handle missing doses in less informative conventional therapeutic drug-monitoring data.

Matched MeSH terms: Cyclosporine/therapeutic use

Characteristics and outcome of autoimmune liver disease in Asian children

Lee WS, Lum SH, Lim CB, Chong SY, Khoh KM, Ng RT, et al.

Hepatol Int, 2015 Apr;9(2):292-302.

PMID: 25788179 DOI: 10.1007/s12072-014-9558-0

BACKGROUND: Little is known about autoimmune liver disease (AILD) in Asian children. We studied the clinical features and predictors of outcome in childhood AILD in an Asian population.

METHODS: Retrospective review of AILD [autoimmune hepatitis type 1 and 2 (AIH1, AIH2), primary sclerosing cholangitis (PSC) and autoimmune sclerosing cholangitis (ASC)] seen at two pediatric liver units in Malaysia.

RESULTS: At presentation, 17 (56%) of the 32 children [19 females, 59%; median (range) age 7.7 (1.8-15.5) years] with AILD (AIH1 = 18, AIH2 = 5, PSC = 0, ASC = 9) had liver cirrhosis. At final review [median (range) duration of follow-up 4.8 (0.4-12) years], 24 patients (75%) survived with a native liver. Twenty-one (66%) were in remission; 19 (AIH1 = 11; AIH2 = 4, ASC = 4) were on prednisolone and/or azathioprine, one on cyclosporine and another on mycophenolate mofetil. Three (AIH1 = 3) were in partial remission. Of the two who underwent liver transplantation (LT; 6.5%; both ASC), one died of primary graft failure after LT. Six patients (19%) died without LT (acute liver failure, n = 1; end-stage liver disease, n = 5). The overall survival rate (native liver and survival post-LT) was 78%. A delay in seeking treatment adversely affected the final outcome [survival with native liver vs. LT or death (duration between onset of disease and treatment; median ± standard error) = 2.5 ± 2.9 months vs. 24.0 ± 13.3 months; p = 0.012].

CONCLUSIONS: Although remission was achieved in the majority of patients with prednisolone and/or azathioprine therapy, delay in seeking diagnosis and treatment adversely affects the outcome of childhood AILD in Malaysia.

Matched MeSH terms: Cyclosporine/therapeutic use

Childhood autoimmune liver disease: indications and outcome of liver transplantation

Chai PF, Lee WS, Brown RM, McPartland JL, Foster K, McKiernan PJ, et al.

J Pediatr Gastroenterol Nutr, 2010 Mar;50(3):295-302.

PMID: 20118802 DOI: 10.1097/MPG.0b013e3181bf0ef7

Graft rejection and disease recurrence are well-recognized complications of liver transplantation (LT) for autoimmune hepatitis (AIH) and autoimmune sclerosing cholangitis (AISC). We describe indications and outcome of LT for childhood AIH and AISC.

Matched MeSH terms: Cyclosporine/therapeutic use

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